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Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature


1 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
2 Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India

Correspondence Address:
Lalita Nemani
Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderbad - 500 082, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.140859

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Year : 2014  |  Volume : 7  |  Issue : 3  |  Page : 221-226

 

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We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.






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1 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
2 Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India

Correspondence Address:
Lalita Nemani
Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderbad - 500 082, Andhra Pradesh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.140859

Rights and Permissions

We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.






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